Helen Keller with ALS

Submitted by aaron on Sat, 2013-10-12 22:35

We have an amazing group of volunteers, who help to make our lives just a little bit more tolerable in the face of the devastation caused by my having ALS. They are coordinated by a smaller group of co-captains who meet occasionally at our house. This afternoon, they met, and asked me as part of the agenda, how was I feeling. I said that I was feeling fine.

I lied.

One of the lesser known facts about ALS is that it, contrary to common wisdom, does have an effect on the brain. I don't know, maybe they say that it doesn't to try to soften the blow when you've been diagnosed with a terminal illness that will slowly rob you of the ability to move a single muscle in your body. "At least his mind will be unaffected."

It turns out that about 40% of ALS patients are afflicted by some cognitive disability, caused by scarring in the frontotemporal lobe. Some 10% experience such an amount of cognitive changes that they are diagnosed with a related illness called Frontotemporal Lobe Degeneration, which ends up resembling dementia. The rest of us experience more minor symptoms, which are normally not even noticed in a clinical setting.

But they are noticed by the families.

At the beginning of my particular journey with ALS, about three years ago, I experienced what I thought at the time was hearing loss. I was not surprised, because my father has always been hard of hearing. Although he attributes it to his nearly thirteen years aboard a submarine with no hearing protection, I figured that it may have had a genetic component. In any case, it had caused just enough conflict in my own home that at the same time I was going to the doctors to find out just why were my arms getting so damned weak, I was also going to an audiologist to find out why I couldn't understand what my wife was saying so frequently.

It turns out that my hearing was fine. Other than a slight loss in upper ranges in my right ear, there was absolutely no problem with my hearing. Except for a slight, inexplicable anomaly: I was unable to understand about twenty-five percent of spoken words. They tried me on hearing aids for a couple of months, but they simply became earwax collectors.

After my diagnosis of ALS, my hearing continued to deteriorate, so back to the audiologist I went. Yet again, my hearing was nearly perfect. Only this time, I was unable to understand fully seventy-five percent of words. According to my wife, this ensued in some hilarity, as the doctor said some things like truck, ice cream, and ball, and I would repeat ridiculous answers such as onions, avocado, and hot dog.

Eventually, Gwen did some research and learned about the potential cognitive losses attributable to ALS. At the top of the list was Receptive Aphasia, which includes the loss of the ability to understand spoken language. My neurologist said that hearing loss is not, no, he said that it is "unequivocally not related to ALS." An assistant of his later admitted that yes, indeed, they have occasionally found patients exhibiting symptoms similar to mine.

So we have adjusted as well as could be expected, given the circumstances. Although it stinks, I have learned that I can sometimes read lips. And the Regal Cinemas have also come to my rescue, offering glasses that project subtitles on the lenses of all their movies. If only life came with subtitles…

But the story doesn't end there. Starting sometime this spring, I learned about another manifestation of my flavor of the curse of Lou Gehrig. For no discernable reason, my eyes shut about ten times an hour, and I am incapable of opening them again of my own volition. My neurologist doesn't know if this is muscular or cognitive. As with the other symptom, this is another rare outcome. About ten percent of patients lose the ability to move their eyes. Although at least he admits that this might be caused by ALS. He told me of a patient who is unable to close his eyes.

To make a long story short, I find myself often trapped in a dark world, filled with mumbling and white noise, as my brain recoils at the sensory deprivation. I found myself at the co-captains' meeting this afternoon, surrounded, blind, by a group of Charlie Brown's teachers, when I was asked how I was feeling. Because it's so difficult to speak, thank you Lou, I lied and said I was fine.

But I'm not feeling fine. I feel sad, and frustrated, and angry, that I am locked out of the world of verbal communication. That I am frequently forbidden from even being a spectator to my life, when my eyes shut unbidden, without an attendant at arm's reach to pry them open. I feel like Helen Keller, but without the benefit of not knowing the difference.

You have every right to be sad, angry and frustrated. This disease is so unfair. You have always been a communicator, since you were a little boy. Even in your crib, you would be up at the crack of dawn, standing up looking out your window and singing with the birds. You learned to talk early, amazed us with your verbal ability, and your quick mind, that, at the age of four, before you were even in school, you recognized numbers way up into the hundreds, as I was reading you a bedtime story from a book with hundreds of pages, and you were pointing to the numbers on the pages and calling out the numbers! I remember I tested you, by flipping through the book, and you still, at random, were able to recognize the numbers

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Thank you so much for sharing while you can. I am a nurse who specializes in ALS. I read this to one of my patients who has had ALS for 36 years and who recently developed FTD! It was shocking to hear her say she forgot something for the first time! She never forgot anything! It took quite a while for the diagnosis since we had not heard of it before. We read your article together and she indicated she could relate to it! Currently, the eyes have been a huge problem. Every day is a guessing game filled with the love we have for her, and she for us! When she is "ON", we are "OFF" and vice versa! It's challenging to say the least. But, we go on living life to the fullest every day and trust in God to lead the way!!

You have every right to be sad, angry and frustrated. This disease is so unfair. You have always been a communicator, since you were a little boy. Even in your crib, you would be up at the crack of dawn, standing up looking out your window and singing with the birds. You learned to talk early, amazed us with your verbal ability, and your quick mind, that, at the age of four, before you were even in school, you recognized numbers way up into the hundreds, as I was reading you a bedtime story from a book with hundreds of pages, and you were pointing to the numbers on the pages and calling out the numbers! I remember I tested you, by flipping through the book, and you still, at random, were able to recognize the numbers.

You have always been an active participant in the world around you, always exploring wherever your eyes led, and your feet followed. When you were little, once, that led to you wandering off, down the block...and a ride in a police car home. You can't imagine how shocked I was for the squad car to pull up in front of the house, and an officer knocking on the door, telling me he had found you down the block, and it was good that you knew your name and where you lived. That wasn't the last time you wandered off.

It breaks my heart, Son of Mine, that you are going through this now. No parent wants to see their child suffer or be sick, and to know that there is no cure, that you have been given a death sentence via a terminal disease, at the prime of life, with so much to live for, and so much to give, so much Love and Light in your heart, is more than I can understand. Yet, even as your body continues its slow journey to dissolution, slowly imprisoning you in the grip of mortality, your mind remains clear, your spirit soars, and you continue to give of yourself, as much as you can. You are truly an inspiration to those around you. In so many ways, you have been a bright, shining Star in this world. I am proud that I was given the blessing of bringing you into the world, as your mother. I love you, Aaron.

That was so beautiful to read... thank you both - Aaron and your Mom!
You do have every right to have and express ALL your feelings and thoughts! Thank you for doing so! I appreciate it much more than, "fine"! You continue to be an inspiration Aaron! And I think you are an even greater inspiration by expressing the full range of your feelings.